tbuckley/cabot-search huggingface dataset:
PB2598: TO INTRODUCE THE CONCEPT OF PATIENT BLOOD MANAGEMENT AND HAEMOVIGILANCE IN GOVERNMENT SECTOR HOSPITALS OF SINDH.
Authors: Samra Waheed
Journal: HemaSphere (2023)

Topic: 31. Transfusion medicine Background: Patient blood management (PBM) encompasses all aspects of the transfusion decision-making process, beginning with the initial patient evaluation and continuing through clinical management. PBM involves the timely, multidisciplinary application of evidence-based medical and surgical concepts aimed at diagnosing and appropriately treating anemia along with minimizing surgical and iatrogenic blood losses and managing coagulopathic bleeding, and supporting the patient while appropriate treatment is initiated Haemovigilance is the set of surveillance procedures covering the entire blood transfusion chain, from the donation and processing of blood and its components, through to their provision and transfusion to patients, and including their follow-up. Aims: In this study, we reviewed the past 3 years performance and data of Regional blood centre, Karachi regarding the concept of patient blood management, transfusion parameters including the C/T ratio, transfusion index and to induce the concept of haemovigilance in the government sector hospitals. This study will allow us to critique as well as appreciate our performance in this regard. Methods: It is a retersopective analysis of our data of previous 3 years. All the data will be taken from BBMIS software (Zaavia), we calculated the Cross match to transfusion ratio and transfusion index of all three years for comparison, we also calcualted the number of requests from surgical ward before the start of Maximum Surgical blood ordering Schedule and after it was implemented to compare the results. Also the reporting of transfusion reactions, will be inculded. All the data was analyzed on SPSS version 23. Results: There was a mark reduction in the CT ratio in the 3 years, starting from 15:1 now to 1.5-1:7:1. after the start of MSBOS, the surgical practices were markedly improved regarding the ordering and wasting of blood. By introducing the concept of blood distribution by blood bank service, the wastage of blood was also markedly reduced. Initially we didnot have any transfusion reaction reported, but after all the awarness sessions and CME, the concept of haemovigilance was improved and the proper reporting of transfusion reactions was started. Summary/Conclusion: : To conclude, Regional blood centre Karachi was able to achieve the better transfusion services to the patients, it was a tough journey, dealing with majority of the illiterate people and with the doctors, who were used to the old blood banking sevices and procedures. However, with the hard work, regular awarness sessions, CME’s, quartely Hosptial transfusion committee meetings and proper distribution of blood products, we have achieved great success in terms of better quality of transfusion services.Keywords: Blood transfusion
P739: INCREASED FOXO1 EXPRESSION IS ASSOCIATED WITH BETTER PROGNOSIS IN MYELODYSPLASTIC SYNDROME PATIENTS RECEIVING DECITABINE THERAPY
Authors: Zheng Zhang, Sida Zhao
Journal: HemaSphere (2023)

Background: Hypomethylating drugs have been widely used in higher risk MDS treatment. However, predictors of treatment response to demethylating drugs have been extensively studied by many investigators based on potential predictors of clinical features, DNA methylation, genetic mutations, gene expression, microRNA, and protein expression, but so far, no consensus has been reached and used in clinical practice. Aims: The aim of our study was to evaluate the role of changes in FOXO1 expression in predicting the response to hypomethylation therapy in myelodysplastic syndromes. Methods: Real-time PCR was used to examine FOXO1 mRNA in 42 patients with de novo MDS who received decitabine. The relationship between FOXO1 gene expression and clinical indicators, cellular immunity, treatment effect and prognosis of patients was also analyzed. Results: The results showed 32 patients with MDS displayed treatment response (more than hematologic improvement) in response to decitabine therapy. MDS patients with treatment response had significantly elevated FOXO1, and elevated FOXO1 was significantly associated not only with increased peripheral blood counts and bone marrow blast cell reduction, but also with amplified T-cell immunity and polarization. Elevated FOXO1 expression and FOXO1 ratio after decitabine were associated with better treatment response. Univariate and multivariate COX analyses showed that induction of elevated FOXO1 expression and FOXO1 ratio were associated with longer overall survival and time to progression. Summary/Conclusion: These results demonstrate that FOXO1 gene enhance treatment response of MDS patients to decitabine via activated adaptive immunity. Keywords: Prognosis, Treatment, MDS
P1218: A REPROGRAMMING OF THE LYMPH NODE MICROENVIRONMENT UNDERLIES LOSS OF COMPARTMENTALISATION IN AGGRESSIVE LYMPHOMAS
Authors: Anna Mathioudaki, Felix Czernilofsky, Lea Jopp-Saile, Raphael Lutz, Dominik Vonflicht, XI Wang, Marc-A. Baertsch, Harald Vohringer, Tobias Roider, Johannes Mammen, Diana Ordonez-Rueda, Caroline Pabst, Wolfgang Huber, Andreas Trumpp, Carsten Müller-Tidow, Gary P. Nolan, Vladimir Benes, Judith B. Zaugg, Daniel Hübschmann, Simon Haas, Sascha Dietrich
Journal: HemaSphere (2023)

Topic: 20. Lymphoma Biology & Translational Research Background: The function of lymph nodes depends on the coordinated structure defined by the lymph node stromal cells (LNSC), which consist of endothelial cells as well as chemokine-secreting mesenchymal cells. In B cell non-Hodgkin lymphoma (B-NHL), the LN structure is disrupted, indicating that LNSCs play a crucial role within the tumor microenvironment (TME). Nevertheless, the molecular mechanisms of stromal reprogramming in B-NHL are not well comprehended. Aims: Here, we studied the LN TME in order to address the long-standing question of why some lymphomas grow in a more organized manner, whereas others grow diffusely. Methods: To understand the molecular pathomechanisms of the LN TME in aggressive lymphomas, we analyzed primary human LN samples of B-NHL different growth patterns (follicular lymphoma, n = 9; diffused large B cell lymphoma, DLBCL, n = 11) as well as non-malignant controls (reactive lymph nodes, n=7) by combining single-cell transcriptomics (scRNA-seq) with multiplexed immunofluorescence imaging (CODEX) together with in vitro co-culture models. Results: Spatial single-cell-resolved analysis allowed fine mapping of LN neighborhoods and compositional changes in lymphomagenesis, including disruption of the vasculature and the expansion of mesenchymal cells. These B-NHL associated LNSCs were characterized by distinct fibrotic and inflammatory signatures as well as dysregulated chemokine gradients, suggesting a phenotypic switch from LN structural organizers to an inflammatory and fibrotic state. In silico cell-cell attraction analysis revealed that the detected chemokine signatures were sufficient to explain the loss of lymph node organization in DLBCL. Furthermore, in vitro co-culture assays using patient-derived LNSCs validated lymphocyte-dependent secretome changes of mesenchymal cells, including elevated expression of inflammatory chemokines. These findings were further corroborated in an independent patient cohort, where we showed that loss of the chemokine expression adequately reflects the extent of the LN’s loss of organization in B-NHL. Summary/Conclusion: Altogether, our findings provide valuable insights into the role of non-hematopoietic cells in the TME and lay a foundation for understanding the structural remodeling in B-NHL at a single-cell and niche-level.Keywords: Diffuse large B cell lymphoma, Non-Hodgkin’s lymphoma, Microenvironment, Follicular lymphoma
PB2596: OPTIMIZING PLATELET TRANSFUSION FOR CLINICAL PRACTICE. DO PLATELETS AGE MATTER FOR THERAPEUTIC PLATELETS TRANSFUSION?
Authors: Samy Attallah, Mohammed Alsharif, Barra Alwali, Foad Alzaharany, Tahani Alzaharany, Zainah Bukhari, Ahmed Salami, Wafa Alanazy, Hawazen Sami, Samah Mokhles, Amany Gusti, Iman Alhazmi
Journal: HemaSphere (2023)

Topic: 31. Transfusion medicine Background: Platelet concentrates (PCs) with the implementation of bacterial testing have a storage period of 7 days. It was reported that platelet function decreased gradually in vitro until the 5th or 7th day of storage. Pooled PCs (PPCs) are mainly used in Europe and Canada and there is the possibility to use many PPCs with 7 days storage for transfusion in case of bleeding or massive transfusion Aims: To test in vitro both storage conditions and function of platelet-rich plasma (PRP-PCs) during storage for 7 days to assess the function of the PCs for the possibility to extend the use of stored PCs up to 7 days and its use for therapeutic and massive platelets transfusion. Methods: In this study 18 PRP-PCs, were evaluated for chemical analysis by measuring pH, LDH production, and glucose consumption levels and platelet aggregation testing using adenosine diphosphate (ADP), collagen (COLL), and ristocetin (RIST) and epinephrine (EPI) on days 1,3,5, and 7 of storage of PCs. Results: The platelets yield was >5.58 x 1010 which indicates accurate platelet separation and storage. The metabolic parameters indicated stable storage conditions throughout the 7-day period. The chemical analysis showed that there is stable pH during all days of storage above 6.4. Aggregation responses of the PCs decreased gradually and significantly (p<0.001) starting from 1st day when compared with 3rd, 5th and7th day. On the 7th day the platelets lost their response to EPI., ADP, with weak response to COLL. while still maintaining a normal response to RIST. Summary/Conclusion: There is a loss of some functions on the 7th day of platelet storage which may indicate that it is suitable for prophylactic more than therapeutic transfusion. In order to provide more clinical benefit to the patients with thrombocytopenic bleeding, the platelets should be less than 7 days of storage.Keywords: Platelet function, Aggregation, Platelet transfusion
PB2556: MEGALOBLASTIC ANEMIA: EPIDEMIOLOGICAL, CLINICAL, AND ETIOLOGICAL PROFILE: ABOUT 200 CASES.
Authors: Kmira Zahra, Zeineb Hasnaoui, Wafa Chenbah, Seif Ncibi, Ouahchi Ines, Nesrine Ben Sayed, Monia Zaier, Yosra Ben Youssef, Haifa Regaieg, Abderrahim Khelif
Journal: HemaSphere (2023)

Topic: 28. Enzymopathies, membranopathies and other anemias Background: Megaloblastic anemias caused by vitamin B 12 and folate deficiencies are the major causes of aregenerative macrocytic anemia. Aims: The aim of our study is to determine the epidemiological, clinical, and etiological profile of megaloblastic anemias (MA). Methods: We retrospectively evaluated 200 patients treated for MA at the department of hematology, Farhat Hached, Sousse, between January 2015 and December 2022. Results: The median age was 45 years (14 - 91) with a sex-ratio M/ F of 0.8. Clinical manifestations were dominated by anemic syndrome in 100% of cases, digestive syndrome in 60% and neuroanemic disorders in 15% of cases. The mean hemoglobin level was 7.5 g/dl and the average MCV was 110 fl (80-141 fl). Leukopenia, thrombocytopenia, and pancytopenia were noted in 48%, 55% and 50% of cases, respectively. A bone marrow aspiration performed in 15% of the cases when the serum vitamin measurement was not possible showed a medullary megaloblastosis. Vitamin B12 deficiency was noted in 180 patients (90%) and folate deficiency in 20 (10%). Out of 100 samples, 60 were positive for the anti-parietal cell antibody test, while out of 50 samples 20 were positive for the anti-intrinsic factor antibody test. An upper gastrointestinal endoscopy performed in 150 patients was normal in 40 patients (26.5%) and showed gastric atrophy in 110 patients (73.5%). Biermer’s disease was the most frequent cause of vitamin B12 deficiency (80% of cases), followed by the non-dissociation of vitamin B12 syndrome in 20% of cases. Malabsorption and drug-related causes were the two causes of folate deficiency found in 60% and 40% of cases, respectively. Summary/Conclusion: Our study showed that the leading cause of vitamin B12 deficiency is Biermer anemia, and the most common cause of folate deficiency is malabsorption. Nutritional deficiencies in vitamin B12 and folate are uncommon. Keywords: Anemia
PB2660: HEREDITARY COAGULATION FACTOR FVII DEFICIENCY CAUSED BY COMPOUND HETEROZYGOUS MUTATIONS
Authors: Rongfu Zhou, Wenjing Gao
Journal: HemaSphere (2023)

Topic: 33. Bleeding disorders (congenital and acquired) Background: Congenital factor (F) VII deficiency is a rare coagulation factor deficiency with an estimated incidence of 1 per 500,000 individuals. Patients with severe FVII deficiency present a broad range of clinical presentations. Aims: To investigate the possible pathogenesis of a patient with PT extension. Methods: The proband’s clinical manifestations, coagulation function test and PT correction test were retrospectively analyzed and the coagulation factor activities and related genes were detected or sequenced. His mother, daughter and elder brother’s activity of coagulation factor and the corresponding gene mutation was also tested. Results: The proband, a 38-year-old male, was presented for repeated and persistent epistaxis, with no abnormal local nasal examination. Coagulation tests showed PT 28s, corrected PT 12s, and normal APTT. FVII: C was 3.8%. NGS with the peripheral blood found that the F7 gene had c.27_28del in Exon1, resulting in heterozygous p.Cys10ProfsTer16, and c.722C> A in Exon 8,causing heterozygous p.Thr241Asn. Sanger sequencing confirmed that the proband’s mother, 61-year-old, had Exon1 heterozygous deletion frameshift mutation c.27_28del in the F7 gene, his daughter, 6 years old, heterozygous c.722C> A missense mutation in Exon 8, and his brother, 40-year-old, had the same two mutations in F7 gene. Summary/Conclusion: The compound heterozygous mutations in the F7 gene might be the molecular pathology of hereditary coagulation factor VII deficiency for the proband. Keywords: Coagulation factors, Gene mutation
Biomechanical origins of proprioceptor feature selectivity and topographic maps in the Drosophila leg
Authors: Akira Mamiya, Anne Sustar, Igor Siwanowicz, Yanyan Qi, Tzu-Chiao Lu, Pralaksha Gurung, Chenghao Chen, Jasper S. Phelps, Aaron T. Kuan, Alexandra Pacureanu, Wei-Chung Allen Lee, Hongjie Li, Natasha Mhatre, John C. Tuthill
Journal: Neuron (2023)
PB2557: CHARACTERISTICS OF AUTOIMMUNE HEMOLYTIC ANEMIA IN ADULTS: A CASE SERIES OF 35 PATIENTS
Authors: Hind Hanani, Layla Kherroubi, Wafa Ammouri, Hajar Khibri, Mouna Maamar, Hicham Harmouche, Mohamed Adnaoui, Zoubida Tazi Mezalek
Journal: HemaSphere (2023)

Topic: 28. Enzymopathies, membranopathies and other anemias Background: Autoimmune hemolytic anemia (AIHA) can be idiopathic or indicative of an underlying pathology. Aims: To analyze epidemiological, clinico-biological, etiological and therapeutic outcomes of AIHA. Methods: Retrospective monocentric study (2017-2022) including patients admitted for hemolytic anemia with a positive direct antiglobulin test and/or cold agglutinins without any other cause. Results: Thirty-five patients were included (28 women, 7 men) with a median age of 47 years [16-90 years]. Clinically, all presented an anemic syndrome with signs of tissue hemolysis. Respectively 22.9% and 2.9% of patients presented with an infectious syndrome and an haemorrhagic syndrome. Median hemoglobin was 5.3 g/dL; 96% presented at least one stigma of biological hemolysis. The direct antiglobulin test was positive in 86.2% (one case of cold agglutinins, 3 cases of mixed autoantibodies). Thirteen patients (37%) had a bone marrow biopsy which contributed to diagnosis in 4 of them. A thoraco-abdomino-pelvic CT scan was performed in 62.9% of patients which showed in half the cases a tumor syndrome (22.7%) or splenomegaly/hepatosplenomegaly (27.3%); it was normal in 50%. AIHA was idiopathic in 32.4% of cases; it was secondary to lupus (23.5%), chronic lymphoid leukemia (17.6%), solid neoplasia (5.9%), Hodgkin’s lymphoma (2.9%), cold agglutinin disease (2.9%) or Evans syndrome (14.7%) in other cases. Ninety-seven percent initially received corticotherapy; 36.4% required rituximab. One death, due to a septic shock, was notified. Summary/Conclusion: The high prevalence of underlying causes of AIHA justifies systematic immunological assessment, serum protein electrophoresis and CT-scan. Despite the effectiveness of corticosteroids, rituximab use is gaining more weight in therapeutic arsenal. Keywords: Autoimmune hemolytic anemia (AIHA)
PB2707: ESMO-MAGNITUDE OF CLINICAL BENEFIT SCALE FOR HAEMATOLOGICAL MALIGNANCIES (ESMO-MCBS:H) VERSION 1.0
Authors: Barbara Kiesewetter, Urania Dafni, Elisabeth G E de Vries, Jorge Barriuso, Giuseppe Curigliano, Veronica González-Calle, Martina Galotti, Bishal Gyawali, Brian Huntly, Ulrich Jaeger, Nicola Jane Latino, Luca Malcovati, Sjoukje Oosting, Gert Ossenkoppele, Martine Piccart, Markus Raderer, Lydia Scarfò, Dario Trapani, Christoph C Zielinski, Ruth Wester, Panagiota Zygoura, Elizabeth Macintyre, Nathan I Cherny
Journal: HemaSphere (2023)

Topic: 36. Ethics and health economics Background: The European Society for Medical Oncology (ESMO) Magnitude of Clinical Benefit Scale (MCBS) has been accepted as a robust tool to evaluate the magnitude of clinical benefit reported in trials for oncological therapies. However, the ESMO-MCBS hitherto has only been validated for solid tumours. With the rapid development of novel therapeutic strategies for haematological malignancies, there is a need to develop an ESMO-MCBS version that is specific for haematological malignancies. Aims: The European Hematology Association (EHA) and ESMO initiated a collaboration to develop a version for haematological malignancies (ESMO-MCBS:H). Methods: The process incorporated five landmarks: field-testing of the ESMO-MCBS v1.1 to identify shortcomings specific to haematological diseases, drafting of the ESMO-MCBS:H forms, peer review and revision of the draft based on re-scoring (resulting in a second draft), assessment of reasonableness of the scores generated, final review and approval by ESMO and EHA including executive boards. Results: Based on the field-testing results of 80 haematological trials and extensive review for feasibility and reasonableness, five amendments to ESMO-MCBS were incorporated in the ESMO-MCBS:H addressing the identified shortcomings. These concerned mainly clinical trial endpoints that differ in haematology versus solid oncology and the very indolent nature of nevertheless incurable diseases such as follicular lymphoma which hampers presentation of mature data. In addition, general changes incorporated in the draft version of the ESMO-MCBS v2 were included and specific forms for haematological malignancies generated. Here we present the final approved version of the ESMO-MCBS:H. Summary/Conclusion: The haematology-specific version ESMO-MCBS:H, allows now full applicability of the scale for evaluating the magnitude of clinical benefit derived from clinical studies in haematological malignancies. Keywords: Myeloma, MDS, Lymphoma, Leukemia
PB2690: CURRENT STATUS AND FACTORS INFLUENCING FEAR OF COVID-19 IN HEMATOPOIETIC STEM CELL TRANSPLANTATION PATIENTS: A CROSS-SECTIONAL STUDY
Authors: Fucong Peng, Huijuan Zhang, Wenjun Xie, Guiying Liu, Qiuhui Zhang, Yuqiu Liu, Wei Guan, Erlie Jiang
Journal: HemaSphere (2023)

Topic: 35. Quality of life and palliative care Background: Patients with hematological malignancies can effectively extend their survival time and improve their quality of life with blood stem cell transplants(HSCT). However, due to pre-treatment and immunosuppressive treatment, patients frequently experience immunodeficiency or a slow recovery of hematopoietic function. As regular epidemic prevention and control in China has been laxened, the risk of COVID-19 has increased, which may make HSCT patients in fear. Aims: This paper aims to investigate the current situation and analyze the associated factors of COVID-19 phobia in HSCT patients. Methods: From January 17 to February 24, 2023, a convenient sampling method was used to investigate adult HSCT patients after transplant at the Stem Cell Transplant Center of Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College. The research tools included fear of corona virus disease 2019 scale (FCV-19S), social support rating scale (SSRS), and hospital anxiety and depression scale(HADS). Results: A total of 168 patients were included in this study, 47.6 percent of thesample were famale. The average age of HSCT recipients was 39.0 (SD=10.9). Seventy-three percent of the HSCT patients were married, and up to 60.71% of the patients reported severe financial burden. The median time after HSCT was 9.00（2.00，23.00）month, and 95.24% of patients received allo-HSCT. Among 168 HSCT patients, 52.98% were infected with COVID-19, and among care-givers, it was 77.38%. The rate of high-level fear was 70.24%, with average score of 20.39 (SD=8.33). (Table 1)Logistic regression showed severe financial burden, previously infected with COVID-19, anxiety and WBC are factors that affect the degree of fear of COVID-19 (P<0.05). The incidence of high levels of fear in anxious patients was 30.78 times higher than in non-anxiety patients(OR=30.78, P<0.05, 95%CI=6.65~142.58). (Table 2) Table 2 Multivariate Analysis (n=168) - Variables β S.E Wals df p OR 95% CI Financial Burden1- -1.638 0.948 2.988 1 0.084 0.194 0.03 1.245 Financial Burden2- -0.282 0.763 0.137 1 0.712 0.754 0.169 3.367 Financial Burden3- -1.087 0.463 5.519 1 0.019 0.337 0.136 0.835 Covid-19 Infected -0.282 0.142 3.943 1 0.047 0.754 0.571 0.996 Anxiety 3.427 0.782 19.197 1 0 30.782 6.645 142.582 WBC 0.22 0.095 5.358 1 0.021 1.247 1.034 1.502 Constant 0.817 1.455 0.315 1 0.574 2.264 Summary/Conclusion: The HSCTpatients are at a high level of fear in COVID-19. The fear level is affected by the financial burden, anxiety, infection experience and the function of the immune system, especiallyanxieties, suggesting that when the prevention and control of the epidemic is laxened, medical staff should pay attention to the mental health of the HSCT patients, so as to alleviate the anguish emotions of patients. Keywords: Adult, HSCT, Risk factor, COVID-19